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Chapter 20: Kidney

Terms in this set (133)

What is nephrotic syndrome?
Excessive glomerular permeability to plasma proteins (massive proteinuria), hypoalbunemia, hyperlipidemia, lipiduria, and generalized edema
What is the most common cause of nephrotic syndrome?
Membranous Nephropathy
What types of antibodies are present in membranous nephropathy?
Antibodies against self-antigens, haptens, and exogenous proteins
T/F: A majority of cases of membranous nephropathy are idiopathic
True
What is membranous nephropathy characterized by?
Diffuse thickening of the glomerular capillary wall and accumulation of immunoglobulin-containing deposits along the subepithelial basement membrane
What stain is used for membranous nephropathy?
Silver stain to demonstrate "spikes" on basement membrane
What do the granular deposits consists of using IF for membranous nephropathy?
IgG and C3
What is the most frequent cause of nephrotic syndrome in children? What ages?
Minimal Change Disease, 2-6 years
What is minimal change disease characterized by?
Diffuse effacement of the foot processes of the visceral epithelial cells but appear virtually normal through light microscopy, no IF IgG and C3
What was minimal change disease historically called and why?
"Lipoid Nephrosis" due to tubular reabsorption of lipoproteins passing through diseased glomeruli
Proteinuria is highly selective for what protein in minimal change?
Albumin
What is a characteristic feature of minimal change regarding treatment?
Dramatic response to corticosteroid therapy
What is the most common cause of nephrotic syndrome in adults in the US?
Focal Segmental Glemerulosclerosis (FSGS)
What is characteristic of FSGS?
Diffuse effacement of foot processes, IF IgG and C3, focal sclerosis and hyalinosis form entrapment of plasma proteins
What clinical signs are seen in FSGS?
Hematuria, Reduced GFR, Hypertension, Non-selective proteinuria, and poor response to corticosteroid therapy
What genes can be altered in FSGS?
Nephrin, Podocin, TRPC6, and CD2AP
What is a variant of FSGS that is a characteristic lesion in HIV-associated nephropathy?
Collapsing Glomerulopathy
What is characteristic of collapsing glomerulopathy?
-Collapse and/or retraction of the entire glomerular tuft, with or without additional FSGS lesions
-Proliferation and hypertrophy of glomerular visceral epithelial cells
What is seen histologically in HIV-associated nephropathy?
Focal, cystic dilation of tubule segments filled with proteinaceous material, inflammation, and fibrosis
What is seen in HIV-associated nephropathy using EM?
Tubuloreticular Inclusions
What is seen in Membranoproliferative Glomerulonephritis (MPGN)?
-Mixture of nephrotic and nephritic
-Large hypercelluar glomeruli with "lobular" appearance
-Proliferation of cells in mesangium and endocapillaries
-Infiltrating leukocytes
What term is used to describe the proliferation of cells predominantly in the mesangium and capillary loops?
Mesangiocapillary Glomerulonephritis
What is seen in silver stain with MPGN?
Glomerular capillary walls with "double contour" or "tram-track" appearance due to new membrane synthesis
What does EM and IF for MPGN Type I reveal?
EM = Discrete subendothelial electron-dense deposits IF = C3 deposits in granular pattern
How does MPGN Type II appear with EM and IF?
EM = "Ribbon-like" GBM with extremely electron dense material
IF = C3 in irregular granular pattern on GBM and C3 present in mesangium in circular aggregates
What are the diabetic nephropathy changes seen?
-Metabolic defect
-Non-enzymatic glycosylation of proteins
-Hemodynamic changes
What is another name for MPGN Type II and what is the abnormality?
Dense Deposit Disease due to excessive activation of the alternative compliment pathway
What are the causes of secondary MPGN Type I?
-Immune Complexes
-alpha-1-AT deficiency
-Malignant Disease
-Hereditary deficiency of compliment regulatory proteins
What lesions are seen in diabetic nephropathy?
- Capillary basement membrane thickening
-Diffuse mesangial sclerosis
-Kimmelstiel-Wilson Disease (PAS pos nodules)
What is seen grossly in diabetic nephrosclerosis?
-Granular surface
-Thin cortex
-Irregular surface
-Cortical Cysts
What is characteristically seen in nephritic syndrome?
-Hematuria (RBC Casts in Urine)
-Elevated Cr
-Oliguria
-Hypertension
-Edema
-Mild proteinuria
What is an immune complex-mediated disorder caused by deposition of Ag-Ab complexes?
Acute Proliferative Glomerulonephritis
Diffuse proliferation/hypercellularity of glomerular cells and leukocytic influx due to immune complexes
Acute proliferative glomerulonephritis disorders
A young child presents with malaise, fever, oliguria, hematuria and cola-colored urine, what disorder is this?
Poststeptococcal Glomerulonephritis
When and what ages does poststreptococcal glomerulonephritis occur?
Occurs 1-4 weeks following strep infection of pharynx or skin (impetigo) in 6-10 year olds
What type of strep is nephritogenic and why?
Group A beta-hemolytic strep due to type 1, 4, and 12 M proteins in cell wall
How do the glomeruli appear in poststreptococcal glomerulonephritis?
Enlarged, hypercellular glomeruli with diffuse infiltration of neutrophils and monocytes and RBC casts
What can be seen with EM in postreptococcal glomerulonephritis?
Discrete electron-dense deposits ("humps")
What are the three types of Rapidly Progressive (Cresentic) Glomerulonephritis?
-Type I (Anti-GBM Antibody IgG)
-Type 2 (Immune Complex)
-Type 3 (Pauci-Immune)
What is characteristic of RPGN?
-Associated with severe glomerular injury
-Rapid and progressive decline in renal function
- > 50% of glomeruli with crescents
How are the cresents formed in RPGN?
Due to accumulation of cells in Bowman's space, fibrin strands are key contributors to crescent formation
What occurs in Goodpasture's syndrome?
Anti-GBM antibodies cross-react with pulmonary alveolar basement membrane causing pulmonary hemorrhage
What is the antigen that is targeted by antibodies in Goodpasture Syndrome?
alpha3 chain of collagen type IV
How is Goodpasture Syndrome treated?
Plasmapheresis to remove autoantibodies; corticosteroids and immunosuppressants
What is seen with IF for Type II RPGN?
Granular "Lumpy Bumpy" Pattern
How do you treat Type II RPGN?
Treat underlying disease, plasmapheresis doesn't help
What are the types of systemic vasculitis that can cause RPGN Type III?
Wegener's (Polyangitis) Granulomatosis and Microscopic polyangitis
What do a majority of patients with RPGN Type III have circulating?
Anti-neutrophil cytoplasmic antibodies (ANCAs)
What do patients with polyangitis granulomatosis have autoantibodies towards?
Proteinase 3
What is a condition of older children and young adults that shows gross hematuria after an infection of the respiratory, GI, or urinary tract?
IgA Nephropathy or Berger Disease
How long does hematuria persist for in patients with IgA Nephropathy?
Hematuria persists for days the subsides and then returns a few months later
What is the pathogenesis of IgA nephropathy?
IgA complexes deposits in mesangium and activate alternative compliment pathway, initiating glomerular injury
What X-linked disease has hematuria that progresses to chronic renal failure accompanied by nerve deafness and eye disorders?
Alport Syndrome
In Alport Syndrome, what do patients have a defective synthesis of? What is this help form?
Type IV Collagen forms GBM, lens of eye, and cochlea
With EM, what is seen in patients with Alport Syndrome?
"Basket-weave" appearance with irregular thickening and thinning of GBM
What disease is characterized by familial asymptomatic hematuria and diffuse thinning of GBM?
Thin Basement Membrane Disease
In Thin Basement Membrane Disease, what is the GBM thickness compared to normal?
In TBMD, GBM is 150-225 nm vs normal 300-400 nm
A patient presents with nephrotic syndrome, the kidneys are enlarged, pale gray, waxy, and firm with deposits in blood vessel walls and kidney interstitium. What condition is this?
Amyloidosis
This lesion is characterized by purpuric skin lesions, abdominal pain, non-migratory arthralgia, and renal abnormalities. What disease is this?
Henoch-Schonlein Purpura
In Henoch-Schonlein Purpura, what deposits in the mesangium of the glomeruli?
IgA
What end-stage glomerular disease develops insidiously and can occur without previous acute disease?
Chronic Glomerulonephritis
What occurs as a result of dialysis?
-Arterial intimal thickening
-Focal calcification
-Deposition of calcium oxalate crystals
-Acquired cystic disease
-Increase risk of renal adenomas and adenocarcinomas
What types of acute kidney injury is characterized by focal epithelial necrosis along nephron with large skip areas?
Ischemic AKI/ATN
Why type of AKI is characterized by extensive necrosis along proximal convoluted tubule?
Nephrotoxic AKI/ATN
What are causes of toxic AKI/ATN?
-Mercuric Chloride
-Carbon Tetrachloride (Dry Cleaning)
-Ethylene Glycol (Ca Oxalate Crystals)
What are the phases of AKI?
-Initiation phase - first 36 hrs, decline in urine output and rise in BUN
-Maintenance phase - sustained decrease in urine output, salt and water overload, rising BUN, metabolic acidosis
-Recovery phase - steady increase in urine volume, hypokalemia, improved BUN and Cr
Most cases of ATN are benign and patients recover, what instances results in a poorer outcome?
-Shock/Sepsis
-Burns
-Multiorgan Failure
What renal disease has a rapid clinical onset, interstistial edema, leukocyte (neutrophils and eosinophils) infiltration of interstitium and tubules?
Acute tubulointerstitial nephritis
What renal disease has mononuclear infiltration, interstitial fibrosis, and widespread tubular atrophy?
Chronic tubulointerstitial nephritis
What is caused by bacterial infection (commonly E. coli) and associated with UTIs?
Acute Pyelonephritis
What can be caused by bacteria, vesicoureteral reflux, or obstruction?
Chronic pyelonephritis
What is seen clinically and microscopically for acute pyelonephritis?
Fever, Dysuria, Costovertebral angle pain, leukocyte casts with patchy intratubular neutrophils
What are some complications of acute pyelonephritis?
-Papillary necrosis
-Pyonephrosis
-Perinephric abcesses
-Renal Scars
-Can progress to sepsis
What is a latent infection in renal allograft recipients which infects tubular epithelial cells, resulting in allograft failure? Treatment?
Polyomavirus Nephropathy, treat by reducing immunosuppresives
What are the hallmarks of chronic pyelonephritis?
-Renal Scarring (Blunted calyx aka broken dorito)
-Chronic tubulointerstitial inflammation
-Dilated, deformed calyces
What are the two forms of chronic pyelonephritis and which is most common?
-Reflux Nephropathy (Most Common)
-Chronic Obstructive Pylonephritis
What renal disease do you see "thyroidization" of the renal tubules?
Chronic Pyelonephritis
What rare from of chronic pyelonephritis, often associated with proteus infection, is characterized by the formation of foamy macrophages intermingled with plasma cells, polymorphonuclear leukocytes, and occasional giant cells?
Xanthogranulomatous pyelonephritis
What renal disease is due to a well recognized adverse reaction to increasing number of drugs resulting in a hypersensitivity reaction 2 weeks after drug exposure?
Acute Drug-Induced Interstitial Nephritis
What renal disease results from excessive analgesic mixtures?
Analgesic Nephropathy
What are the steps for pathogenesis of analgesic nephropathy?
Papillary Necrosis and Tubulointerstitial nephritis
What do patients with analgesic nephropathy have a small risk of developing?
Transitional Papillary Carcinoma
What renal disease results from uric acid crystals precipiated in tubules and collecting ducts causing obstruction, usually due to chemotherapy?
Acute Urate Nephropathy
What renal disease results from high doses of oral phosphate solutions, typically due to colonscopy prep solutions, causing renal insufficiency?
Acute Phosphate Nephropathy
What renal disease results from hyperuricemia associated with gout, causing tubular obstruction?
Chronic Urate Nephropathy
Term for uric acid stones in the kidney
Nephrolithiasis
What renal diease is caused by bence jones porteinuria in patients with multiple myeloma and results in renal insufficiency?
Plasma Cell Myeloma
What portion of the kidney recieves 90% of renal cirulation?
Cortex
What renal diease is associated with diabetes mellitus and hypertension, causing hardening ischemic atrophy of nephrons, intesitial fibrosis, and glomerulosclersosis?
Benign Nephroclerosis
In benign nephrosclerosis, plasma proteins add to the wall and GBM
Hyaline Arteriosclerosis
In benign nephrosclerosis, large muscular arteries with medial and intimal thickening
Fibroelastic hyperplasia
What is associated with accelerated hypertension, intimal hyperplasia demonstrating hyperplastic arteriopathy (onion-skinning) and has an increased risk of presenting in young black males?
Malignant nephrosclerosis
This is secondary to hypertension, caused by atheromatous plaque at origin of renal artery or fibromuscular dysplasia of renal artery, bruit is present
Renal Artery Stenosis
Thrombotic microangiopathy caused by what organism results in hemolytic uremic syndrome?
E. coli 0157:H7 which synthesizes Shiga-like toxin
What is an inherited or acquired deficiency of ADAMTS13 which regulate vWF function?
Thrombotic thrombocytompenic purpura
What is an inherited mutation/autoantibodies of/to completment regulatory proteins?
Atypical Hemolystic Uremic Syndrome
What disease occurs during manipulation of severely diseased aorta where cholesterol crystals embolize from atheromatous plaque?
Atheroembolic renal disease
What is the most common cause of renal infarcts?
Left atrial or ventricular mural thrombi
Kidney low or pelvic in location
Ectopic
Kidney fused at lower or upper poles, anterior to aorta and inferior vena cava
Horseshoe Kidney
Failure of kidenys to develop to normal size
Hypoplasia
Failure of the kideny to from
Agenesis
What congenital kidney disease is multicystic, extermely irregular, may present as a flank mass and is associated with other genitourinary anomalies?
Multicystic Renal Dysplasia
Dialation of renal pelvis and calyces due to increased renal pelvis pressue from obstruction
Hydronephrosis
Where do most renal stones arise from?
Kidney
What are the types of renal stones? Most common?
-Calcium (most common)
-Triple Stone
-Uric Acid
-Cystine
What type of stone is associated with bacterial infection with proteus?
Triple (Struvite) Stones
What stones form from genetic defects in renal amino acid absorption?
Cystine stones
What kidney disease has high penetrance with massively enlarged kidneys, mass of cysts that can arise in all parts of the nephron with functioning nephrons between cysts?
Autosomal Dominant (Adult) Polycystic Kidney Disease
What is the most common gene mutated in Autosomal Dominant Polycystic Kidney Disease? What is the protein?
PKD1, polycystin 1
What protein is encoded by PDK2 and what is it's function?
Polycystin 2, is a cation channel, mutation disrupts regulation of intracelluar calcium
What extrarenal congenital anomalies tend to be present with adult polycystic kidney diease?
-Polycystic Liver Diease
-Intracranial berry aneurysms
-Mitral valve prolase
What is the most common cause of death in adult polycystic kidney diease?
Coronary artery diease and hypertensive heart disease
What rare, autosomal recessive kidney diease in children which can develop into congential heptic fibrosis and dilates all collecting ducts?
Autosomal recessive (childhood) polycystic kidney disease
What gene is mutated in childhood polycystic kidney disease? What protein does it encode?
PKHD1, encode fibrocystin
What kidney disease presents in adults with multiple dilations in the medullary collecting ducts and can predispose patients to renal calculi?
Medullary Sponge Kidney
What kidney diease onsets in childhood, causes cysts in the medulla, and causes polyuria, sodium wasting, and tubular acidosis?
Nephronophthisis
What genes code for nephrocystin and are associated with primary cilia in nephronophthisis?
NPH1 and NPH3
What gene codes for inversin and mediates right-left embryologic patterning in nephronophthisis?
NPH2
From what do patients develop acquired cystic diease and what are they at increased risk for?
Dialysis-associated, renal cell carcinoma
What are the benign renal tumors?
-Papillary adenoma
-Angiomyolipoma
-Oncocytoma
What benign renal tumor is well-circumscribed, < 5 mm, originates from tubular epithelium, and associated with trisomies 7 and 17?
Renal Papillary Adenoma
What benign renal tumor consists of blood vessel, smooth muscle, and adipose tissue and is seen in 50% of patients with tuberous sclerosis?
Angiomyolipoma
What benign renal tumor originates from intercalated cell of the collecting duct, is mahogany brown, well encapsulated, and demonstrates numeous mitochonria in EM?
Oncocytoma
What is the most common renal cell carcinoma?
Clear cell carcinoma
What mutation is present in clear cell carcinoma?
3p tumor supressor sequences in VHL gene
What RCC is common in dialysis-associated diease, not assocaited with 3p deletion but with MET proto-oncogene?
Papillary RCC
What RCC originates from the intercalated cells of collecting duct, dains with Hale's Colloidal Iron, and has pale eosinophilic cytoplasm and perinuclear halo?
Chrompophobe RCC
What is the classic triad of RCC?
-Costovertebral pain
-Papable abdominal mass
-Hematuria
CPT Codes: Kidney Bx
88305
CPT Codes: Kidney Partial/Total Nephrectomy
88307
CPT Codes: Ureter Bx
88305
CPT Codes: Ureter Resection
88307
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